Highly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells

Friedreich ataxia is an inherited neurodegenerative disease that leads to progressive disability. There is currently no effective treatment and patients die prematurely. The underlying genetic defect leads to reduced expression of the mitochondrial protein frataxin. Frataxin insufficiency causes mit...

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Bibliografiske detaljer
Udgivet i:Neurobiol Dis
Main Authors: Rufini, Alessandra, Cavallo, Francesca, Condò, Ivano, Fortuni, Silvia, De Martino, Gabriella, Incani, Ottaviano, Di Venere, Almerinda, Benini, Monica, Massaro, Damiano Sergio, Arcuri, Gaetano, Serio, Dario, Malisan, Florence, Testi, Roberto
Format: Artigo
Sprog:Inglês
Udgivet: Academic Press 2015
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4358773/
https://ncbi.nlm.nih.gov/pubmed/25549872
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2014.12.011
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